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Acute Disseminated Encephalomyelitis in Children

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Acute Disseminated Encephalomyelitis in Children Acute Disseminated Encephalomyelitis in Children S. N. Krishna Murthy, MD*‡§; Howard S. Faden, MD‡ʈ; Michael E. Cohen, MD*‡§; and Rohit Bakshi, MD*¶# ABSTRACT. Objective. To describe the epidemio- cute disseminated encephalomyelitis logic, clinical, neuroimaging, and laboratory features; (ADEM) is considered a monophasic acute treatment; and outcome in a cohort of children with acute demyelinating disorder of the central ner- disseminated encephalomyelitis (ADEM). A vous system (CNS) characterized by diffuse neuro- Methods. A 6-year retrospective chart review of chil- logic signs and symptoms coupled with evidence of dren with the diagnosis of ADEM was conducted. multifocal lesions of demyelination on neuroimag- Results. Eighteen cases were identified. Sixteen pa- tients (88%) presented in either winter or spring. Thir- ing. The epidemiology of ADEM has changed since 1 teen children (72%) had a recent upper respiratory tract its original description by Lucas in the early 18th illness. Patients presented most often with motor deficits century. At that time, ADEM commonly followed (77%) and secondly with altered consciousness (45%). common childhood infections such as measles, Spinal fluid abnormalities occurred in 70%. Despite rig- smallpox, and chickenpox and was associated with orous microbiologic testing, a definite microbiologic di- significant mortality and morbidity. In a series of agnosis was established only in 1 child with Epstein-Barr case reports in 1931 in The Lancet, McAlpine2 de- virus disease and probable or possible diagnoses in 3 scribed 3 sets of patients with ADEM: 1) postvacci- children with Bartonella henselae, Mycoplasma pneu- nation, 2) after infectious fevers such as in measles, moniae, or rotavirus disease. Brain magnetic resonance and 3) spontaneous. Those with spontaneous and imaging identified lesions in the cerebral cortex in 80%, in subcortical white matter in 93%, in periventricular postvaccination ADEM did well despite the lack of white matter in 60%, in deep gray matter in 47%, and in antibiotics, steroids, and intensive care facilities, brainstem in 47% of patients. Eleven patients (61%) were whereas those with an infectious cause fared poorly. treated with corticosteroids, and 2 were treated with in- A number of recent reports of ADEM in children travenous immunoglobulins. All patients survived. have confirmed the observations of McAlpine.3,4 Three patients (17%) had long-term neurologic sequelae. Several articles suggested that improved outcome of Conclusions. Epidemiologic evidence from this study ADEM was attributable mainly to the use of steroids; suggests an infectious cause for ADEM. The agent is however, evidence for this was mainly anecdotal.5,6 most likely a difficult-to-diagnose winter/spring respira- The purpose of the present study was to review tory virus. Magnetic resonance imaging was the neuro- ADEM from a single institution with an emphasis on imaging study of choice for establishing the diagnosis and for following the course of the disease. Prognosis for the relationship of clinical features, microbiology, survival and outcome was excellent. Recurrent episodes neuroimaging, and treatment to clinical outcome. of ADEM must be differentiated from multiple sclerosis. Eighteen patients with ADEM were identified. Re- Pediatrics 2002;110:e0–e0. URL: www.pediatrics.org/cgi/ spiratory infections preceded the neurologic presen- doi/10.1542/peds.; acute disseminated encephalomyelitis, tation in the vast majority. Although in most cases a ADEM, encephalitis, postinfectious encephalitis, encepha- specific cause could not be identified, the outcome lomyelitis. was good regardless of treatment. ABBREVIATIONS. ADEM, acute disseminated encephalomyelitis; METHODS CNS, central nervous system; MRI, magnetic resonance imaging; The inpatient database of Children’s Hospital of Buffalo was FLAIR, fluid-attenuated inversion recovery; PCR, polymerase broadly searched for patients with the diagnosis of ADEM, viral chain reaction; EBV, Epstein-Barr virus; Ig, immunoglobulin; TR, encephalitis, postinfectious encephalitis, encephalomyelitis, and repetition time; TE, echo time; NSA, number of signal averages; transverse myelitis. Sixty-seven cases that occurred between Jan- FOV, field of view; CT, computerized tomography; WBC, white uary 1995 and March 2001 were identified. The diagnosis of blood cell; CSF, cerebrospinal fluid; IVIG, intravenous gamma- ADEM was based on the acute onset of neurologic signs and globulin; MS, multiple sclerosis. symptoms together with magnetic resonance imaging (MRI) evi- dence of multifocal, hyperintense lesions on fluid-attenuated in- version recovery (FLAIR) and T2-weighted images. Of the 67 From the Departments of *Neurology and ‡Pediatrics, State University of patients, 18 patients fulfilled the diagnostic criteria for ADEM. New York at Buffalo, School of Medicine and Biomedical Sciences, Buffalo, Clinical information was obtained from the inpatient case New York; Divisions of §Child Neurology and ࿣Infectious Diseases, Chil- records. Microbiologic data were extracted from laboratory re- dren’s Hospital of Buffalo, Buffalo, New York; and ¶Imaging Services and ports and progress notes in the individual charts. Records main- the #Buffalo Neuroimaging Analysis Center, Jacobs Neurological Institute tained in the microbiology laboratories were reviewed for any of Kaleida Health, Buffalo, New York. tests performed beginning 1 month before admission to the hos- Received for publication Dec 18, 2001; accepted Apr 8, 2002. pital and ending 1 month after discharge. The specific tests re- Reprint requests to (H.S.F.) Division of Infectious Diseases, Children’s viewed included cultures for bacteria, viruses, and fungi; fluores- Hospital of Buffalo, 219 Bryant St, Buffalo, NY 14222. E-mail: cent antibody tests for respiratory viruses; polymerase chain [email protected] reaction (PCR) tests for enteroviruses, herpes simplex virus, Ep- PEDIATRICS (ISSN 0031 4005). Copyright © 2002 by the American Acad- stein-Barr virus (EBV), and Mycoplasma pneumoniae; enzyme- emy of Pediatrics. linked immunosorbent assay for rotavirus; and immunoglobulin http://www.pediatrics.org/cgi/content/full/110/2/Downloaded from www.aappublications.org/newse1 by guestPEDIATRICS on September 24, Vol. 2021 110 No. 2 August 2002 e1 G (IgG) and IgM antibody tests for viruses and M pneumoniae.To TABLE 1. Clinical Features of ADEM in 18 Patients ascertain the clinical relevance of the microbiologic test results, we interpreted them in relation to the history of the present illness, n (%) the medical history, and the physical examination. In the case of Systemic signs and symptoms antibody titers, IgM-specific antibody levels, rising IgG-specific Fever 7 (38.5) antibody levels, or relatively high single IgG-specific antibody Nausea and/or vomiting 5 (27.5) levels were considered significant. An infectious diagnosis was Headache 4 (22.5) classified as definite, probable, possible, or not diagnostic on the Stiff neck 1 (5.5) basis of the interpretation of the microbiologic test results. Neurological signs and symptoms All head MRI scans were performed with a uniform protocol on Motor deficits* 14 (77) an inpatient 1.5-T unit (Philips Gyroscan ACS-NT, Best, the Neth- Altered consciousness 8 (44.5) erlands). The protocol included axial conventional spin-echo T1- Sensory deficits 5 (27.5) weighted images before and after a single dose (0.1 mmol/kg) of Urinary symptoms 5 (27.5) gadolinium contrast (repetition time/echo time [TR/TE]: 450/20, Cranial neuropathy† 4 (22.5) ϫ 5-mm thickness, 0.5-mm gaps, 205 256 matrix size, number of Seizures 3 (16.5) signal averages [NSA] 1, field of view [FOV] 23 cm, scanning time Nystagmus 2 (11) 2:59), axial fast spin-echo T2-weighted images (TR/TE: 5000/100, Internuclear ophthalmoplegia 2 (11) ϫ 6-mm, 0.6-mm gaps, 245 256, NSA 3, FOV 23 cm, 15 echoes, Aphasia 1 (5.5) scanning time 3:20), and axial fast spin-echo FLAIR images (TR/ TE/TI: 8000/120/2300, 5-mm, 1.0-mm gaps, 140 256, NSA 2, FOV * Ataxia (7), paraparesis (4), hemiparesis (2), and monoparesis (1). 23 cm, 21 echoes, scanning time 3:12). Sagittal and coronal T1 and † Optic, oculomotor, abducens, and facial nerves. FLAIR images were also performed. Diffusion-weighted imaging and magnetization transfer imaging were not performed. Spinal MRI of the cervical and thoracic cord included T1- and T2- history of upper respiratory tract illness 2 days to 4 weighted axial and sagittal and postcontrast T1 imaging. The hard copies of brain and spine MRI and brain computerized tomogra- weeks before presentation. An average of 10 days phy (CT) scans of all patients were obtained and reread by an occurred between the upper respiratory tract illness experienced neuroimager without knowledge of clinical involve- and the appearance of neurologic symptoms. None ment (R.B.). All studies were available for review except for MRI of the patients had vaccinations in the 3 months of the head of 2 patients and of the spine of 1 patient. The imaging before presentation. Details of the clinical presenta- assessment included quantification of T2/FLAIR lesions and their size and location and the presence of mass effect and enhance- tion are presented in Table 1. Nonspecific signs or ment. Spinal MRI scans available for 5 patients were also re- symptoms of systemic illness, such as fever, head- viewed. Follow-up scans were reviewed for degree of improve- ache, nausea, and vomiting, occurred in 74% of pa- ment, new lesions, and enhancement. Detailed neuroimaging
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